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Article ; Online: ADAMTS13 Biomarkers in Management of Immune Thrombotic Thrombocytopenic Purpura.

Sui, Jingrui / Zheng, Liang / Zheng, X Long

Archives of pathology & laboratory medicine

2022 Volume 147, Issue 8, Page(s) 974–979

Abstract: Context.—: Immune thrombotic thrombocytopenic purpura (iTTP) is a rare but potentially fatal blood disorder resulting from acquired deficiency of plasma ADAMTS13, a metalloprotease that cleaves endothelium-derived ultralarge von Willebrand factor. ... ...

Abstract	Context.—: Immune thrombotic thrombocytopenic purpura (iTTP) is a rare but potentially fatal blood disorder resulting from acquired deficiency of plasma ADAMTS13, a metalloprotease that cleaves endothelium-derived ultralarge von Willebrand factor. Standard of care for iTTP including therapeutic plasma exchange, caplacizumab, and immunosuppressives, known as triple therapy, has led to a significant reduction in the disease-related mortality rate. The first International Society of Thrombosis and Haemostasis TTP guideline stresses the importance of having plasma ADAMTS13 activity testing in the algorithm for diagnosis and management of iTTP. However, the predictive role of assessing plasma ADAMTS13 activity and inhibitors or other ADAMTS13-related parameters in patients with acute iTTP and during remission has not been systematically evaluated. Objective.—: To review and assess the predictive values of testing plasma ADAMTS13 activity, antigen, and inhibitors or anti-ADAMTS13 immunoglobulin G at various stages of disease in outcomes of iTTP. Data sources.—: Peer-reviewed publications and personal experience. Conclusions.—: We conclude that assessing ADAMTS13 biomarkers is not only essential for establishing the initial diagnosis, but also crucial for risk stratification and the early detection of disease recurrence. This may guide therapeutic interventions during acute episodes and for long-term follow-up of iTTP patients.
MeSH term(s)	Humans ; ADAMTS13 Protein ; Biomarkers ; Immunosuppressive Agents ; Purpura, Thrombotic Thrombocytopenic/diagnosis ; Purpura, Thrombotic Thrombocytopenic/therapy ; Thrombosis ; von Willebrand Factor
Chemical Substances	ADAMTS13 Protein (EC 3.4.24.87) ; Biomarkers ; Immunosuppressive Agents ; von Willebrand Factor
Language	English
Publishing date	2022-10-08
Publishing country	United States
Document type	Journal Article ; Review
ZDB-ID	194119-7
ISSN	1543-2165 ; 0363-0153 ; 0096-8528 ; 0003-9985
ISSN (online)	1543-2165
ISSN	0363-0153 ; 0096-8528 ; 0003-9985
DOI	10.5858/arpa.2022-0050-RA
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Article ; Online: Cerebral Infarction in Immune Thrombotic Thrombocytopenic Purpura Is Associated with Old Age, Hypertension, Smoking, and Anti-ADAMTS13 Ig, But Not with Mortality.

Memon, Raima / Sui, Jingrui / Lin, Chen / Zheng, X Long

TH open : companion journal to thrombosis and haemostasis

2021 Volume 5, Issue 1, Page(s) e1–e7

Abstract: ... ...

Abstract	Background
Language	English
Publishing date	2021-01-13
Publishing country	Germany
Document type	Journal Article
ZDB-ID	2901738-5
ISSN	2512-9465 ; 2567-3459
ISSN (online)	2512-9465
ISSN	2567-3459
DOI	10.1055/s-0040-1722610
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Article ; Online: Acute Respiratory Distress Syndrome in a Patient With Acute Promyelocytic Leukemia: Overlapping Between Differentiation Syndrome and COVID-19.

Sui, Jingrui / Kelmenson, Daniel / Hu, Shimin / Cao, Liyun

Journal of hematology

2021 Volume 10, Issue 5, Page(s) 217–220

Abstract: Differentiation syndrome (DS) is a relatively common and severe complication in acute promyelocytic leukemia (APL) patients undergoing induction therapy with all-trans retinoic acid (ATRA) and/or arsenic trioxide (ATO). DS is a multisystem disorder with ... ...

Abstract	Differentiation syndrome (DS) is a relatively common and severe complication in acute promyelocytic leukemia (APL) patients undergoing induction therapy with all-trans retinoic acid (ATRA) and/or arsenic trioxide (ATO). DS is a multisystem disorder with pulmonary involvement. The coronavirus disease 2019 (COVID-19) caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection is also a systemic disorder with similar pulmonary and other clinical manifestations as DS. Here, we report an APL case with overlapping between DS and COVID-19. After admission to the hospital, the patient was diagnosed with APL and underwent differentiation therapy with ATRA/ATO. In the meantime, COVID-19 was diagnosed with a positive polymerase chain reaction test of SARS-CoV-2 from an oropharyngeal swab. The patient developed acute respiratory distress syndrome, coagulopathy, and acute kidney injury, which fit the clinical pictures of both DS and COVID-19. The patient died at last and this complicate case imposed big challenges for clinicians due to the laboratory and imaging findings of DS disguised in the context of COVID-19. Therefore, comprehensive treatment strategy should be considered to balance the risk and benefit of differentiation therapy in the context of COVID-19.
Language	English
Publishing date	2021-09-15
Publishing country	Canada
Document type	Case Reports
ZDB-ID	2662519-2
ISSN	1927-1220 ; 1927-1220
ISSN (online)	1927-1220
ISSN	1927-1220
DOI	10.14740/jh904
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Article ; Online: Elevated plasma levels of syndecan-1 and soluble thrombomodulin predict adverse outcomes in thrombotic thrombocytopenic purpura.

Lu, Ruinan / Sui, Jingrui / Zheng, X Long

Blood advances

2021 Volume 4, Issue 21, Page(s) 5378–5388

Abstract: Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a potentially fatal blood disorder resulting from acquired deficiency of plasma ADAMTS13 activity. Despite recent advances in early diagnosis and novel therapeutics, the mortality rate of ... ...

Abstract	Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a potentially fatal blood disorder resulting from acquired deficiency of plasma ADAMTS13 activity. Despite recent advances in early diagnosis and novel therapeutics, the mortality rate of acute iTTP remains as high as 10% to 20%. Moreover, a reliable clinical and laboratory parameter that predicts disease severity and outcomes is lacking. We show in the present study that plasma levels of syndecan-1 (Sdc-1) and soluble thrombomodulin (sTM) on admission were dramatically increased in patients with acute iTTP and remained substantially elevated in a subset of patients compared with healthy controls. The elevated admission plasma levels of Sdc-1 and sTM were associated with abnormal Glasgow coma scale scores, low estimated glomerular filtration rates, the need for intensive care, and in-hospital mortality rates. Moreover, a further simultaneous increase in plasma Sdc-1 and sTM levels at the time of clinical response/remission (eg, when normalization of platelet counts and substantial reduction of serum lactate dehydrogenase activity were achieved) was highly predictive of iTTP recurrence. These results demonstrate that endothelial injury, resulting from disseminated microvascular thromboses, is severe and persistent in patients with acute iTTP. Plasma levels of Sdc-1 and sTM on admission and in remission are predictive of in-hospital mortality and recurrence of acute iTTP, respectively. Thus, an incorporation of such novel plasma biomarkers into the risk assessment in acute iTTP may help implement a more vigorous and intensive therapeutic strategy for these patients.
MeSH term(s)	Humans ; Plasma ; Purpura, Thrombocytopenic, Idiopathic ; Purpura, Thrombotic Thrombocytopenic ; Syndecan-1 ; Thrombomodulin
Chemical Substances	Syndecan-1 ; Thrombomodulin
Language	English
Publishing date	2021-03-26
Publishing country	United States
Document type	Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't
ZDB-ID	2915908-8
ISSN	2473-9537 ; 2473-9529
ISSN (online)	2473-9537
ISSN	2473-9529
DOI	10.1182/bloodadvances.2020003065
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Article ; Online: Elevated Plasma Fibrinogen Is Associated With Excessive Inflammation and Disease Severity in COVID-19 Patients.

Sui, Jingrui / Noubouossie, Denis F / Gandotra, Sheetal / Cao, Liyun

Frontiers in cellular and infection microbiology

2021 Volume 11, Page(s) 734005

Abstract: Background: The coronavirus disease-19 (COVID-19) is characterized with intense inflammatory response, cardiac involvement, and coagulopathy. Fibrinogen, as a biomarker for inflammation, cardiovascular disease, and coagulation, has not been fully ... ...

Abstract	Background: The coronavirus disease-19 (COVID-19) is characterized with intense inflammatory response, cardiac involvement, and coagulopathy. Fibrinogen, as a biomarker for inflammation, cardiovascular disease, and coagulation, has not been fully investigated yet. The aim of this study was to assess the clinical application of fibrinogen in COVID-19 patients. Methods: We retrospectively analyzed the demographic and laboratory characteristics of 119 COVID-19 patients in the University of Alabama of Birmingham Medical Center. Correlations of fibrinogen on admission with intensive care unit (ICU) admission, disease severity, and laboratory parameters were analyzed. Results: Among the 119 COVID-19 patients, 77.3% (92/119) had severe disease, and 59.5% (71/119) patients were admitted to the ICU. Elevated fibrinogen was detected in 67.2% (80/119) of the patients. Fibrinogen levels were significantly associated with inflammatory markers and disease severity, but not with cardiac injury biomarker high sensitivity troponin I. Patients with severe disease had increased fibrinogen levels upon admission compared to patients with non-severe disease ( Conclusions: Fibrinogen is commonly elevated in COVID-19 patients, especially in those with severe disease. Elevated fibrinogen correlates with excessive inflammation, disease severity, and ICU admission in COVID-19 patients.
MeSH term(s)	COVID-19 ; Fibrinogen ; Humans ; Inflammation ; Retrospective Studies ; SARS-CoV-2 ; Severity of Illness Index
Chemical Substances	Fibrinogen (9001-32-5)
Language	English
Publishing date	2021-08-03
Publishing country	Switzerland
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	2619676-1
ISSN	2235-2988 ; 2235-2988
ISSN (online)	2235-2988
ISSN	2235-2988
DOI	10.3389/fcimb.2021.734005
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Article ; Online: Identification of Biomarkers in Patients with Thrombotic Thrombocytopenic Purpura Presenting with Large and Small Ischemic Stroke.

Lin, Chen / Memon, Raima / Sui, Jingrui / Zheng, X Long

Cerebrovascular diseases extra

2021 Volume 11, Issue 1, Page(s) 29–36

Abstract: Background: Thrombotic thrombocytopenic purpura (TTP) is a rare blood disorder resulting in organ damage including ischemic strokes. We sought to characterize the neuroimaging patterns of stroke in a large cohort of patients with immune-mediated TTP ( ... ...

Abstract	Background: Thrombotic thrombocytopenic purpura (TTP) is a rare blood disorder resulting in organ damage including ischemic strokes. We sought to characterize the neuroimaging patterns of stroke in a large cohort of patients with immune-mediated TTP (iTTP) and determined their associations with clinical and laboratory parameters and outcomes. Methods: We analyzed the Alabama TTP Registry who had laboratory confirmation of acute iTTP. We reviewed the neuroimaging patterns of those with ischemic stroke on MRI, clinical information, and laboratory results. Small ischemic strokes were ≤20 mm in their maximum diameter in the axial plane. Large ischemic strokes were >20 mm. Student t test, Mann-Whitney U test, and χ2 test were all used for data analysis. Results: Of 108 iTTP patients, 21 had ischemic stroke on neuroimaging. The median platelet count in these patients was 12 × 109/L (interquartile range, IQR, 8.8-21 × 109/L), plasma ADAMTS13 activity 1.8 U/dL (IQR 0-4.5 U/dL), and the mean plasma level of anti-ADAMTS13 IgG was 6,595.8 U/mL (SD 3,448.9 U/mL). Comparison between patients with large ischemic strokes (n = 10) and small ischemic strokes (n = 11) revealed that patients with small stroke were older (p = 0.043) and had higher plasma levels of citrullinated histone 3 (p = 0.006) and histone/DNA complex (p = 0.014) than those with large strokes. There were no significant differences between 2 stroke groups in mortality or exacerbation. Conclusions: iTTP patients can present with large ischemic strokes and are usually younger. Further research should be performed in assessing different etiologies of iTTP-associated stroke based on neutrophil extracellular trap formation biomarkers (e.g., histone markers) seen in small ischemic stroke.
MeSH term(s)	ADAMTS13 Protein/blood ; ADAMTS13 Protein/immunology ; Adult ; Alabama ; Autoantibodies/blood ; Biomarkers/blood ; Cell-Free Nucleic Acids/blood ; Citrullination ; DNA/blood ; Extracellular Traps/metabolism ; Female ; Histones/blood ; Humans ; Ischemic Stroke/diagnostic imaging ; Ischemic Stroke/etiology ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Platelet Count ; Predictive Value of Tests ; Purpura, Thrombotic Thrombocytopenic/blood ; Purpura, Thrombotic Thrombocytopenic/complications ; Purpura, Thrombotic Thrombocytopenic/diagnosis ; Registries ; Retrospective Studies ; Risk Factors
Chemical Substances	Autoantibodies ; Biomarkers ; Cell-Free Nucleic Acids ; Histones ; DNA (9007-49-2) ; ADAMTS13 Protein (EC 3.4.24.87) ; ADAMTS13 protein, human (EC 3.4.24.87)
Language	English
Publishing date	2021-02-18
Publishing country	Switzerland
Document type	Comparative Study ; Journal Article
ZDB-ID	2651613-5
ISSN	1664-5456 ; 1664-5456
ISSN (online)	1664-5456
ISSN	1664-5456
DOI	10.1159/000513574
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Article: Cerebral Infarction in Immune Thrombotic Thrombocytopenic Purpura Is Associated with Old Age, Hypertension, Smoking, and Anti-ADAMTS13 Ig, But Not with Mortality

Memon, Raima / Sui, Jingrui / Lin, Chen / Zheng, X. Long

TH Open

2021 Volume 05, Issue 01, Page(s) e1–e7

Abstract: Background: Neurological involvement is common in patients with immune thrombotic thrombocytopenic purpura (iTTP), but the frequency, risk factors, and outcomes of these with imaging-confirmed stroke in iTTP are not known.: Methods: We selected 66 ... ...

Abstract	Background: Neurological involvement is common in patients with immune thrombotic thrombocytopenic purpura (iTTP), but the frequency, risk factors, and outcomes of these with imaging-confirmed stroke in iTTP are not known. Methods: We selected 66 out of 109 iTTP patients with neurological signs and symptoms and reviewed their CT/MRI (computed tomography/magnetic resonance imaging) findings for the evidence of stroke and other clinical information in Alabama TTP Registry. Results: Of these, 52 (78.8%) had their CT/MRI done on admission in whom 22 (42.3%) were positive for multiple acute or chronic infarcts. The patients with image-confirmed ischemic stroke were older, and appeared to be associated with a history of hypertension and smoking. Additionally, patients with imaging-confirmed stroke showed higher plasma concentrations of anti-ADAMTS13 IgG than those without stroke. More interestingly, there was no statistically significant difference in the rate of exacerbation and 60-day mortality between those with and without stroke. Conclusion: Ischemic cerebral infarcts are common findings in brain imaging studies of patients with acute iTTP; old age, chronic hypertension, and smoking, as well as high plasma concentrations of anti-ADAMTS13 IgG may be the potential risk factors for cerebral infarction in these patients. The presence of image-confirmed ischemic stroke, however, does not predict exacerbation and 60-day mortality, although the long-term effect of such ischemic brain damage on cognitive function and quality of life remains to be determined.
Keywords	thrombotic thrombocytopenic purpura ; inflammatory mediators ; ADAMS/ADAMTS13 ; von Willebrand factor ; cerebrovascular disease ; autoimmune diseases ; autoantibodies
Language	English
Publishing date	2021-01-01
Publisher	Georg Thieme Verlag KG
Publishing place	Stuttgart ; New York
Document type	Article
ZDB-ID	2901738-5
ISSN	2512-9465 ; 2567-3459
ISSN (online)	2512-9465
ISSN	2567-3459
DOI	10.1055/s-0040-1722610
Database	Thieme publisher's database

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Article ; Online: Plasma levels of S100A8/A9, histone/DNA complexes, and cell-free DNA predict adverse outcomes of immune thrombotic thrombocytopenic purpura.

Sui, Jingrui / Lu, Ruinan / Halkidis, Konstantine / Kocher, Nicole K / Cao, Wenjing / Marques, Marisa B / Zheng, X Long

Journal of thrombosis and haemostasis : JTH

2021 Volume 19, Issue 2, Page(s) 370–379

Abstract: Background: Immune thrombotic thrombocytopenic purpura (iTTP) is a life-threatening blood disorder, primarily resulting from autoantibodies against ADAMTS13. Infection or inflammation often precedes acute iTTP. However, the association of inflammation ... ...

Abstract	Background: Immune thrombotic thrombocytopenic purpura (iTTP) is a life-threatening blood disorder, primarily resulting from autoantibodies against ADAMTS13. Infection or inflammation often precedes acute iTTP. However, the association of inflammation and inflammatory mediators with disease severity and outcome of acute iTTP is not fully assessed. Objectives: Here, we determined plasma levels of S100A8/A9, histone/DNA complexes, citrullinated histone H3 (CitH3), and cell-free DNA (cfDNA) in a cohort of 108 acute episodes from 94 unique iTTP patients and healthy controls, and assessed the association of each of these biomarkers with the disease severity and mortality. Results: All acute iTTP patients had significantly increased plasma levels of S100A8/A9 (median 84.8, interquartile range [IQR] 31.2-157.4 µg/mL), histone/DNA complexes (median 55.7, IQR 35.8-130.8 U/mL), CitH3 (median 3.8, IQR 2.2-6.4 ng/mL), and cfDNA (median 937.7, IQR 781.3-1420.0 ng/mL) on the admission blood samples when compared with healthy controls. An increased plasma level of S100A8/A9, histone/DNA complex and cfDNA was associated with organ damage, coagulopathy, and mortality in iTTP. After being adjusted for age and history of hypertension, Cox proportional hazard regression analysis demonstrated that a hazard ratio (95% confidence interval) for an elevated plasma level of S100A8/A9, histone/DNA complexes, and cfDNA was 11.5 (1.4-90.9) (P = .021), 10.3 (2.7-38.5) (P = .001), and 12.8 (3.9-42.0) (P = .014), respectively. Conclusion: These results indicate that inflammation or plasma inflammatory mediators such as S100A8/A9 or NETosis markers such as histone/DNA complexes and cfDNA may play a role in pathogenesis of iTTP, which may help stratify patients with a high risk of death during acute iTTP episodes.
MeSH term(s)	Calgranulin A/blood ; Calgranulin B/blood ; Cell-Free Nucleic Acids/blood ; DNA ; Histones ; Humans ; Purpura, Thrombocytopenic, Idiopathic/diagnosis ; Purpura, Thrombotic Thrombocytopenic/diagnosis
Chemical Substances	Calgranulin A ; Calgranulin B ; Cell-Free Nucleic Acids ; Histones ; S100A8 protein, human ; S100A9 protein, human ; DNA (9007-49-2)
Language	English
Publishing date	2021-01-03
Publishing country	England
Document type	Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't
ZDB-ID	2112661-6
ISSN	1538-7836 ; 1538-7933
ISSN (online)	1538-7836
ISSN	1538-7933
DOI	10.1111/jth.15176
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Zs.A 5805: Show issues			Location: Je nach Verfügbarkeit (siehe Angabe bei Bestand) bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular Jg. 1995 - 2021: Lesesall (2.OG) ab Jg. 2022: Lesesaal (EG)
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Article ; Online: NPM1A

Sun, Chengming / Gao, Yujie / Yang, Liping / Shao, Huiyuan / Li, Jie / Gao, Xuejun / Ma, Li / Lin, Mingming / Sui, Jingrui

Open life sciences

2018 Volume 13, Page(s) 236–241

Abstract: Objective: The aim of the study was to investigate whether nucleophosmin type A mutation (: Methods: Plasma : Results: Plasma levels of : Conclusions: Results reveal ... ...

Abstract	Objective: The aim of the study was to investigate whether nucleophosmin type A mutation ( Methods: Plasma Results: Plasma levels of Conclusions: Results reveal that
Language	English
Publishing date	2018-08-21
Publishing country	Poland
Document type	Journal Article
ZDB-ID	2817958-4
ISSN	2391-5412 ; 2391-5412
ISSN (online)	2391-5412
ISSN	2391-5412
DOI	10.1515/biol-2018-0028
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Article ; Online: NPM1A in plasma is a potential prognostic biomarker in acute myeloid leukemia

Sun Chengming / Gao Yujie / Yang Liping / Shao Huiyuan / li Jie / Gao Xuejun / Ma Li / Lin Mingming / Sui Jingrui

Open Life Sciences, Vol 13, Iss 1, Pp 236-

2018 Volume 241

Abstract: The aim of the study was to investigate whether nucleophosmin type A mutation (NPM1A) in plasma was associated with the prognosis of patients with acute myeloid leukemia (AML). ...

Abstract	The aim of the study was to investigate whether nucleophosmin type A mutation (NPM1A) in plasma was associated with the prognosis of patients with acute myeloid leukemia (AML).
Keywords	acute myeloid leukemia ; npm1a ; prognosis ; biomarker ; Biology (General) ; QH301-705.5
Language	English
Publishing date	2018-08-01T00:00:00Z
Publisher	De Gruyter
Document type	Article ; Online
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